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Plexiform neurofibromatosis of penis: a rare presentation of type 1 neurofibromatosis
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  • Published on:
    Response to eletter
    • Ravi Banthia, Doctor Urology and Renal Transplantation, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
    Thanks for the comments on our manuscript entitled "Plexiform neurofibromatosis of penis: a rare presentation of type 1 neurofibromatosis."
     
    We think that this is a very good suggestion for treating such cases. Selumetinib has been found to be effective to treat neurofibromatosis type 1 in children 2 years of age and older. It is an inhibitor of mitogen-activated protein kinase and has been recommended as a first-line therapy approved for paediatric neurofibromatosis patients who have inoperable and bulky lesions. 
    Selumetinib therapy was a good option for this particular child but there were several reasons to choose surgery for this patient. Firstly the deformity was unsightly and grotesque considering the almost double length of the penis was leading to social discrimination, peer pressure and solitary life for this child. The patient has been rehabilitated with just one surgical operation in which after debulking the penile size is within socially acceptable limits. S...
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    Conflict of Interest:
    None declared.
  • Published on:
    Selumetinib should be preferred to a surgical approach in case of extensive plexiform neurofibromas
    • Francesco Baldo, Pediatric Resident University of Trieste
    • Other Contributors:
      • Laura Morra, Pediatric Resident
      • Andrea Magnolato, Pediatrician
      • Egidio Barbi, Pediatrician

    Dear Editor,
    we found the case report of Banthia et al. extremely interesting. First of all, penile plexiform neurofibromas are quite infrequent. Secondly, the subject described did not have a former clinical diagnosis of neurofibromatosis type I prior to the identification of this tumor, which is even rarer. However, we take exception to the therapeutic approach chosen by the authors. In fact, the surgical treatment of deep and extended plexiform neurofibromas is generally unsatisfactory, since their complete resection is frequently unattainable, allowing the masses to grow back (1-2). A partial tumor debulking should be considered only in case of high-risk conditions, such as a urethral or ureteral compression or a bowel obstruction. Apart from these scenarios, a medical approach should always be preferred, at least as a first attempt.
    Selumetinib, an inhibitor of MEK 1 and 2 kinase, has demonstrated to be effective in reducing the size of plexiform neurofibromas in pediatric patients (3-4). The drug is generally well tolerated and safe in the pediatric population. Only few patients failed to show a clinical response to the treatment and even fewer had to stop it due to the appearance of severe adverse events.
    In a recent study, we reported a cohort of nine patients with inoperable plexiform neurofibromas treated with selumetinib (5). Eight of them showed a partial response to the drug, i.e. a reduction of the tumor size by more than 20%. Remarkably, on...

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    Conflict of Interest:
    None declared.